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THE EYELID FORMATION IN CASE OF THE ABLEPHARON-MACROSTOMIA SYNDROME

Abstract

The ablepharon-macrostomia syndrome is an extremely rare genetic pathology which causes serious craniofacial deformations and other numerous anomalies of the face, genitals and skin. It refers to the ectodermal dysplasia and is inherited in an autosomal-dominant manner. The reference literature regarding this condition is scarce.Purpose: to describe a case of surgical formation of eyelids in a girl with aplasia of the eyelids in both eyes with ablepharon-macrostomy syndrome (AMS).Material and methods. In this article, we report on the case of plastic surgery to form the eyelids in a girl with ablepharon-macrostomy syndrome.Results and discussion. Based on the child's clinical data, a multistage formation of the eyelids with a free skin flap and with theuse of the Alloplant biomaterial was carried out. Subsequently, the palpebral fissure was lengthened with an optical purpose. Expansion of the palpebral fissure in the subsequent periods was not performed because of the danger of lagophthalmos development. Further surgical correction of the patient's eyelids was postponed until the formation of the facial skeleton.Conclusions: AMS treatment should be directed to the elimination of the specific symptoms that appear. Surgical correction should be carried out in stages, taking into account the growth of the facial skeleton. Thus, in order to carry out the maximum complete treatment of the AMS, the collaborative work of many specialists is required.

About the Authors

N. D. Kul'baev
ФГБУ «Всероссийский центр глазной и пластической хирургии» Минздрава России
Russian Federation


E. P. Solov'eva
ФГБУ «Всероссийский центр глазной и пластической хирургии» Минздрава России
Russian Federation


References

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Review

For citations:


Kul'baev N.D., Solov'eva E.P. THE EYELID FORMATION IN CASE OF THE ABLEPHARON-MACROSTOMIA SYNDROME. Bashkortostan Medical Journal. 2021;16(4):46-49. (In Russ.)

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ISSN 1999-6209 (Print)